Interleukin-1 in Febrile Infection-Related Epilepsy Syndrome

Febrile infection-related epilepsy syndrome (FIRES) characteristically affects previously healthy children, who experience a sudden and explosive onset of super-refractory status epilepticus preceded by febrile infection accompanied fulminant neurogenic inflammation. FIRES, however, can affect individuals all ages is subcategory new-onset refractory epilepticus. This definition FIRES excludes in infants. rare type epileptic encephalopathy with rapidly progressive seizures devastating prognosis, as drug-resistant often follows without latency period. Although the exact pathogenesis has not been elucidated, functional deficiency endogenous interleukin-1 receptor antagonist implicated genetic predisposition to FIRES. Dysregulation interleukin-1β–interleukin-1 1 (IL-1β–IL-1R1) signaling pathway appears be involved In this review, authors summarize IL-1β–IL-1R1 signaling, nucleotide-binding oligomerization domain NLRP3 inflammasome, IL-1 targeted therapy for